Otosclerosis presenting in children and adolescents is uncommon. Typically, otosclerosis presents as a slowly progressive conductive hearing loss in the third to fourth decade, Many well-documented studies have demonstrated excellent long-term hearing results with stapedectomy. Although stapedectomy is highly effective, the rare complications may be devastating. Thirty-one patients, 21 years or younger, underwent 40 stapedectomies for otosclerosis at our institution, The average age at surgery was 16 years, with a range of 7 to 21 years, Postoperative results showed an average improvement in air-bone gap of 22 dB, Clinical and audiologic data were collected over a mean follow-up period of 25 years, There was no statistically significant difference in air-bone gap when comparing the immediate postoperative gap (measured 2 months after stapedectomy) with the last gap recorded (mean, 25 years after stapedectomy), Fifty percent of the ears operated on maintained an air-bone gap within 10 dB at the last follow-up, and an additional 40% maintained a 10- to 20-dB gap, There were no significant relationships between demographic or clinical factors and ''success'' (gap less than or equal to 10 dB) or long-term gap closure, Our data demonstrate that stapedectomy is an effective method for closing the air-bone gap in children and adolescents with otosclerosis, and long-term results parallel those of adults, showing maintenance of excellent gap closure. This review represents the largest population with the longest follow-up in children who underwent stapedectomy for the treatment of otosclerosis.